TY - JOUR
T1 - Risk factors for mortality and circulatory outcome among neonates prenatally diagnosed with ebstein anomaly or tricuspid valve dysplasia
T2 - A multicenter study
AU - Freud, Lindsay R.
AU - McElhinney, Doff B.
AU - Kalish, Brian T.
AU - Escobar-Diaz, Maria C.
AU - Komarlu, Rukmini
AU - Puchalski, Michael D.
AU - Jaeggi, Edgar T.
AU - Szwast, Anita L.
AU - Freire, Grace
AU - Levasseur, Stéphanie M.
AU - Kavanaugh-Mchugh, Ann
AU - Michelfelder, Erik C.
AU - Moon-Grady, Anita J.
AU - Donofrio, Mary T.
AU - Howley, Lisa W.
AU - Tierney, Elif Seda Selamet
AU - Cuneo, Bettina F.
AU - Morris, Shaine A.
AU - Pruetz, Jay D.
AU - van der Velde, Mary E.
AU - Kovalchin, John P.
AU - Ikemba, Catherine M.
AU - Vernon, Margaret M.
AU - Samai, Cyrus
AU - Satou, Gary M.
AU - Gotteiner, Nina L.
AU - Phoon, Colin K.
AU - Silverman, Norman H.
AU - Tworetzky, Wayne
N1 - Publisher Copyright:
© 2020 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.
PY - 2020/11/3
Y1 - 2020/11/3
N2 - BACKGROUND: In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. METHODS AND RESULTS: This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia. Among live-born patients, clinical and echocardiographic factors were evaluated for association with neonatal mortality and palliated versus biventricular circulation at discharge. Of 176 live-born patients, 7 received comfort care, 11 died <24 hours after birth, and 4 had insufficient data. Among 154 remaining patients, 38 (25%) did not survive to discharge. Nearly half (46%) underwent intervention. Mortality differed by procedure; no deaths occurred in patients who underwent right ventricular exclusion. At discharge, 56% of the cohort had a biventricular circulation (13% following intervention) and 19% were palliated. Lower tricuspid regurgitation jet velocity (odds ratio [OR], 2.3 [1.1–5.0], 95% CI, per m/s; P=0.025) and lack of antegrade flow across the pulmonary valve (OR, 4.5 [1.3–14.2]; P=0.015) were associated with neonatal mortality by multivariable logistic regression. These variables, along with smaller pulmonary valve dimension, were also associated with a palliated outcome. CONCLUSIONS: Among neonates with Ebstein anomaly or tricuspid valve dysplasia diagnosed in utero, a variety of management strategies were used across centers, with poor outcomes overall. High-risk patients with low tricuspid regurgitation jet velocity and no antegrade pulmonary blood flow should be considered for right ventricular exclusion to optimize their chance of survival.
AB - BACKGROUND: In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. METHODS AND RESULTS: This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia. Among live-born patients, clinical and echocardiographic factors were evaluated for association with neonatal mortality and palliated versus biventricular circulation at discharge. Of 176 live-born patients, 7 received comfort care, 11 died <24 hours after birth, and 4 had insufficient data. Among 154 remaining patients, 38 (25%) did not survive to discharge. Nearly half (46%) underwent intervention. Mortality differed by procedure; no deaths occurred in patients who underwent right ventricular exclusion. At discharge, 56% of the cohort had a biventricular circulation (13% following intervention) and 19% were palliated. Lower tricuspid regurgitation jet velocity (odds ratio [OR], 2.3 [1.1–5.0], 95% CI, per m/s; P=0.025) and lack of antegrade flow across the pulmonary valve (OR, 4.5 [1.3–14.2]; P=0.015) were associated with neonatal mortality by multivariable logistic regression. These variables, along with smaller pulmonary valve dimension, were also associated with a palliated outcome. CONCLUSIONS: Among neonates with Ebstein anomaly or tricuspid valve dysplasia diagnosed in utero, a variety of management strategies were used across centers, with poor outcomes overall. High-risk patients with low tricuspid regurgitation jet velocity and no antegrade pulmonary blood flow should be considered for right ventricular exclusion to optimize their chance of survival.
KW - Congenital heart disease
KW - Ebstein anomaly
KW - Mortality
KW - Neonate
KW - Outcome
KW - Palliation
KW - Right ventricle
KW - Tricuspid regurgitation
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U2 - 10.1161/JAHA.120.016684
DO - 10.1161/JAHA.120.016684
M3 - Article
C2 - 33076749
AN - SCOPUS:85095671040
SN - 2047-9980
VL - 9
JO - Journal of the American Heart Association
JF - Journal of the American Heart Association
IS - 21
M1 - e016684
ER -